Abstracts

Neonatal epileptic encephalopathy with suppression-bursts (SB) often renders impression of intractability especially in patients who have pre-existing brain damage with spasm, psychomotor deterioration and so-called hypsarrhythmia. Therefore, treatment strategy is commonly underestimated. We explored the situation from the admitted children. And we documented two patients who underwent multiple subpial transactions for revision of diagnosis after delicate assessment of seizures. A 1-year-10-months-old and a 3-year-old boy had habitual seizures because of cerebral infection (seizure for one year) and unknown etiology (seizures since born) respectively. Refractory infantile spasms were diagnosed for manifestations of neck flexion, extension of upper limbs, complex rotation of the eyes and nystagmoid movements; and paroxysms of generalized high-voltage slow waves and spikes. Both boys had partial effective to the anti-epileptic agents and adrenocorticotropic hormone (ACTH). But partial seizure was concerned for the disclosure of adversion, ipsilateral turning of eyes in the former, and breath spells in the latter preceding the spasms. The runs of focal rhythmic spikes in the video-EEG recordings, and the benefit of carbamazepine administration tended to think of partial seizure in addition to infantile spasms. The decrease of metabolism in the corresponding cortical region of possible epileptogenesis by the interictal 2-deoxy-2[18F]fluoro-D-glucose (FDG) positron emission tomography (PET) helped the localization of surgical intervention. Guided by the intraoperative electrocorticography, multiple subpial transections were performed on the right frontal gyri in the young boy and the left frontotemporal gyri in the elder. Over half a year following, there was marked reduction of seizure numbers (mean seizure numbers per day: from 270.0 [preoperatively] to 31.5 [6 months postoperatively] in younger body; from 54.5 [preoperatively] to 14.7 [4 months postoperatively] in elder body; [italic]p[/italic][lt]0.001) and spare of the hemiparesis. In addition, compromised consciousness was prevented by withdrawal of antiepileptic agents. Although the neonatal convulsions with SB pattern or hypsarrhythmia commonly indicate unfavored outcome, the failure of the response to pharmacological measures is still indicative of precise observation of seizure manifestations, correction of diagnosis, and consideration of surgical possibility. With regard to the good response of carbamazepine and the surgical outcome in these 2 boys, our experiences enforced the assertion that for neonatal epilepsy with suppression-burst or hypsarrhythmic pattern, revision of diagnosis should be considered as soon as treatment with drug fails.